Article ; Online: Embryonal tumor with multilayered rosettes: Post-treatment maturation and implications for future therapy.
Cancer reports (Hoboken, N.J.)
2023 Volume 6, Issue 5, Page(s) e1812
Abstract: Background: Embryonal tumor with multilayered rosettes (ETMR) is a deadly grade IV pediatric brain tumor. Despite an intensive multimodal treatment approach that includes surgical resection, high-dose chemotherapy, and radiotherapy, the progression-free ...
Abstract | Background: Embryonal tumor with multilayered rosettes (ETMR) is a deadly grade IV pediatric brain tumor. Despite an intensive multimodal treatment approach that includes surgical resection, high-dose chemotherapy, and radiotherapy, the progression-free survival at 5 years is less than 30%. Case: We report a case of long-term survival in a 5-month old female with a large mass in the posterior fossa, diagnosed as ETMR, which subsequently underwent treatment-induced maturation. Prior to chemotherapy, histopathology revealed an abundance of highly proliferative, undifferentiated cells and multilayered rosette structures. Conversely, post-treatment histopathology revealed cell populations that differentiated into neuronal and ganglionic phenotypes. At 5-year follow-up, the patient remains progression-free. Conclusion: This finding contributes to the few reports to date of post-treatment differentiation/maturation of ETMR cell populations, with an implication for less cytotoxic therapeutic interventions aimed at differentiation. |
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MeSH term(s) | Humans ; Female ; Central Nervous System Neoplasms ; Neoplasms, Germ Cell and Embryonal/therapy ; Brain Neoplasms/pathology ; Neuroectodermal Tumors, Primitive/genetics ; Neuroectodermal Tumors, Primitive/pathology ; Neuroectodermal Tumors, Primitive/therapy |
Language | English |
Publishing date | 2023-03-25 |
Publishing country | United States |
Document type | Case Reports ; Research Support, Non-U.S. Gov't |
ISSN | 2573-8348 |
ISSN (online) | 2573-8348 |
DOI | 10.1002/cnr2.1812 |
Database | MEDical Literature Analysis and Retrieval System OnLINE |
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